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Natural history of chronic thromboembolic pulmonary disease with no or mild pulmonary hypertension.

Authors :
Reddy SA
Swietlik EM
Robertson L
Michael A
Boyle S
Polwarth G
Screaton NJ
Ruggiero A
Nethercott SL
Taboada D
Sheares KK
Hadinnapola C
Cannon JE
Bunclark K
Jenkins D
Ng C
Toshner MR
Pepke-Zaba J
Source :
The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation [J Heart Lung Transplant] 2023 Sep; Vol. 42 (9), pp. 1275-1285. Date of Electronic Publication: 2023 May 16.
Publication Year :
2023

Abstract

Background: We describe baseline characteristics, disease progression and mortality in chronic thromboembolic pulmonary disease patients as a function of mean pulmonary artery pressure (mPAP) according to new and previous definitions of pulmonary hypertension.<br />Methods: All patients diagnosed with chronic thromboembolic pulmonary disease between January, 2015 and December, 2019 were dichotomized according to initial mPAP: ≤ 20 mmHg ('normal') vs 21-24 mmHg ('mildly-elevated'). Baseline features were compared between the groups, and pairwise analysis performed to determine changes in clinical endpoints at 1-year, excluding those who underwent pulmonary endarterectomy or did not attend follow-up. Mortality was assessed for the whole cohort over the entire study period.<br />Results: One hundred thirteen patients were included; 57 had mPAP ≤ 20 mmHg and 56 had mPAP 21-24 mmHg. Normal mPAP patients had lower pulmonary vascular resistance (1.6 vs 2.5WU, p < 0.01) and right ventricular end-diastolic pressure (5.9 vs 7.8 mmHg, p < 0.01) at presentation. At 3 years, no major deterioration was seen in either group. No patients were treated with pulmonary artery vasodilators. Eight had undergone pulmonary endarterectomy. Over 37 months median follow-up, mortality was 7.0% in the normal mPAP group and 8.9% in the mildly-elevated mPAP group. Cause of death was malignancy in 62.5% of cases.<br />Conclusions: Chronic thromboembolic pulmonary disease patients with mild pulmonary hypertension have statistically higher right ventricular end-diastolic pressure and pulmonary vascular resistance than those with mPAP ≤ 20 mmHg. Baseline characteristics were otherwise similar. Neither group displayed disease progression on non-invasive tests up to 3 years. Mortality over 37 months follow-up is 8%, and mainly attributable to malignancy. Further prospective study is required to validate these findings.<br /> (Copyright © 2023 International Society for the Heart and Lung Transplantation. All rights reserved.)

Details

Language :
English
ISSN :
1557-3117
Volume :
42
Issue :
9
Database :
MEDLINE
Journal :
The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation
Publication Type :
Academic Journal
Accession number :
37201688
Full Text :
https://doi.org/10.1016/j.healun.2023.04.016